The Early History of Arimoclomol for Inclusion Body Myositis


  • Mazen M. Dimachkie
  • Pedro M. Machado
  • Claus Sundgreen
  • Thomas Blaettler
  • Jeffery Statland
  • Andrew Heim
  • Linda Greensmith
  • Michael G. Hanna
  • Richard J. Barohn



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Cudkowicz ME, Shefner JM, Simpson E, Grasso D, Yu H, Zhang H, Shui A, Schoenfeld D, Brown RH, Wieland S, Barber JR; Northeast ALS Consortium. Arimoclomol at dosages up to 300 mg/day is well tolerated and safe in amyotrophic lateral sclerosis. Muscle Nerve. 2008;38(1):837-44. doi: 10.1002/mus.21059. PMID: 18551622.

Kieran D, Kalmar B, Dick JR, Riddoch-Contreras J, Burnstock G, Greensmith L. Treatment with arimoclomol, a coinducer of heat shock proteins, delays disease progression in ALS mice. Nat Med. 2004;10(4):402-5. doi: 10.1038/nm1021. Epub 2004 Mar 21. PMID: 15034571.

Ahmed M, Machado PM, Miller A, Spicer C, Herbelin L, He J, Noel J, Wang Y, McVey AL, Pasnoor M, Gallagher P, Statland J, Lu CH, Kalmar B, Brady S, Sethi H, Samandouras G, Parton M, Holton JL, Weston A, Collinson L, Taylor JP, Schiavo G, Hanna MG, Barohn RJ, Dimachkie MM, Greensmith L. Targeting protein homeostasis in sporadic inclusion body myositis. Sci Transl Med. 2016;8(331):331ra41. doi: 10.1126/scitranslmed.aad4583. PMID: 27009270; PMCID: PMC5043094.

Jackson CE, Barohn RJ, Gronseth G, Pandya S, Herbelin L; Muscle Study Group. Inclusion body myositis functional rating scale: a reliable and valid measure of disease severity. Muscle Nerve. 2008;37(4):473-6. doi: 10.1002/mus.20958. PMID: 18236463.

Study of Arimoclomol in Inclusion Body Myositis. Trial details publicly available at:




How to Cite

Dimachkie, M. ., Machado, P., Sundgreen, C. ., Blaettler, T. ., Statland, J. ., Heim, A. ., Greensmith, L. ., Hanna, M. ., & Barohn, R. (2021). The Early History of Arimoclomol for Inclusion Body Myositis. RRNMF Neuromuscular Journal, 2(2).



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